J Korean Neurol Assoc.  1991 Sep;9(3):362-366.

A Case of Wilson's Disease Associated with Somatomotor Status Epileticus

• Jung C
• Park MY
• Byun YJ
• Park CS

Abstract

Wilson's disease is a genetically transmitted autosomal recessive disorder of copper metabolism which affects the liver. Brain and other organs. The seizure in Wilson's disease is not infrequent but. It's report is very rare in Korea. The frequency of seizure is about 10 times more than that of general population The statistical proportions of types of seizure associated with Wilson's disease do not differ significantly from that seen in the general population. And the most common seizure type is focal motor form. The issue of the mechanisms producing seizure in Wilson's disease has attracted controversy, but the cause of seizure seems to be related with copper itself. Although the seizure is well controlled by anticonvulsants, the prognosis of seizure depends on the treatment of Wilson`s disease. We report a case of Wilson's disease associated with somatomotor status epilepticus.

MeSH Terms

Anticonvulsants
Brain
Copper
Hepatolenticular Degeneration*
Korea
Liver
Metabolism
Prognosis
Seizures
Status Epilepticus
Anticonvulsants
Copper